The UMD-FBN1 mutations database
Record ID: 83

Mutation description


Variation name (cDNA level)Variation name (protein level)Variation statusVariation class
c.3391A>Tp.Asn1131TyrHeterozygousMutation

wt codonwt aamutant codonmutant aamutational eventmutation type
AACAsnTACTyrA->TTv

StructureKey Residue (HCD)Pyrimidin doubletCpG
cb EGF-like #13 Ca2+ bindingYes, non coding strandNo

Mutation impact


At the mRNA levelOn restriction map
NANew restriction site(s): none
Lost restriction site(s): none

Conservation (0-1)SIFT (0-1)UMD-predictor (0-100)
0.670.00 (pathogenous)100 (Pathogenous)

Patient and sample data


Sample IDPatient statusGenderTransmissionAge of onsetAge of deathGeographic origin
USA02OMA F0006 I498ProbandNAde novo?U.S.A

Phenotypic groupDisease
NANeonatal MFS

Clinical data


Symptom
no clinical data

Reference


Reference IDPubMed IDReference
359101298
Wang M, Wang JY, Cisler J, Imaizumi K, Burton BK, Jones MC, Lamberti JJ, Godfrey M. "Three novel fibrillin mutations in exons 25 and 27: classic versus neonatal Marfan syndrome". Hum Mutat 1997;9(4):359-62.